This article is part of the supplement: 2011 Pediatric Rheumatology Symposium: Abstracts

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Organ system-involvement in SLE and relationship with demographic factors, disease duration and health-related quality of life in childhood SLE

Lakshmi N Moorthy14*, Maria J Baratelli10, Margaret GE Peterson3, Afton L Hassett15, Alexa B Adams3, Laura V Barinstein5, Emma J MacDermott3, Elizabeth C Chalom8, Karen Onel12, Linda I Ray16, Jorge Lopez-Benitez9, Christina Pelajo9, Kathleen A Haines2, Daniel J Kingsbury4, Victoria W Cartwright4, Philip J Hashkes7, Nora G Singer6, Gina A Montealegres11, Ingrid Tomanova-Soltys11, Andreas O Reiff1, Sandy D Hong13 and Thomas JA Lehman3

  • * Corresponding author: Lakshmi N Moorthy

Author Affiliations

1 Childrens Hospital LA, Los Angeles, CA, USA

2 Hackensack University Medical Center, Hackensack, NJ, USA

3 Hospital for Special Surgery, New York, NY, USA

4 Legacy Emanuel Children's Hospital, Portland, OR, USA

5 Maimonides Medical Center, Brooklyn, NY, USA

6 MetroHealth Medical Center, Cleveland, OH, USA

7 Shaare Zedek Medical Center, Jerusalem, Israel, USA

8 St. Barnabas Medical Center, New Brunswick, NJ, USA

9 Tufts Medical Center, Boston, MA, USA

10 UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA

11 University Hospitals Case Medical Center, Cleveland, OH, USA

12 University of Chicago, Chicago, IL, USA

13 University of Iowa Children's Hospital, Iowa City, IA, USA

14 University of Medicine and Dentistry of NJ (UMDNJ)- Robert Wood Johnson Medical School, New Brunswick, NJ, USA

15 University of Michigan Medical School, Ann Arbor, MI, USA

16 University of Mississippi Medical Center, Jackson, MS, USA

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Pediatric Rheumatology 2012, 10(Suppl 1):A22  doi:10.1186/1546-0096-10-S1-A22

The electronic version of this article is the complete one and can be found online at:

Published:13 July 2012

© 2012 Moorthy et al; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Damage in childhood systemic lupus erythematosus (SLE) affects ocular, musculoskeletal, neuropsychiatric, renal, cardiovascular, peripheral vascular, and skin domains and damage can affect their health related quality of life (HRQOL). Aggressive treatments have improved survival in childhood SLE, but the disease is still associated with significant morbidity. The objective of our multicenter study is to examine the organ system-involvement in childhood SLE and the relationship of damage with HRQOL, age, gender, ethnicity and disease duration.


In this cross-sectional study, children ≤18 years with SLE and parents completed the Simple Measure of the Impact of Lupus Erythematosus in Youngsters© (SMILEY©) and physicians measured Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI). SMILEY© is a new, brief, 24-item HRQOL assessment tool for pediatric SLE, that has recently been validated in US English. The four domains are: Effect on self, Limitations, Social and Burden of SLE. Responses are in the form of a 5-faces scale for easy comprehension. Higher percentage scores indicate better HRQOL. Contingent upon the data distribution of the above variables, we used student t-test, Mann-Whitney U test or the Kruskal-Wallis (KW) test to examine the relationship of SDI with age, gender, ethnicity, disease duration and HRQOL.


Out of a total of 169 children (17% male), 59 children (35%) had any damage (SDI score>0). Their age, damage and disease duration and specific organ-system involvement is given in table 1. Their ethnicities were: Black (39%), Asian or Pacific Islander (12%), Latino (27%), White (18%) and other (5%). Children predominantly had renal, neuropsychiatric, skin, and musculoskeletal involvement. Significant difference was found in damage with disease duration (p=0.005, Mann Whitney U test). There was no significant difference in damage in patients with different gender, ages or ethnicity. Parent SMILEY© total and all domain scores were decreased in patients with damage compared to patients without damage (table 2), but only the Effect on self domain scores was statistically significant (p=0.02). The child SMILEY© total, Limitation and Effect on self domain scores were lower in patients who had any damage.

Table 1. SDI scores and organ-system involvement, and HRQOL scores

Table 2. Means of parent total and domain scores of SMILEY for patients with no damage (SDI=0) and patients with any damage (SDI>0)


Damage in childhood SLE is significantly related to disease duration. Renal, neuropsychiatric, skin, and musculoskeletal systems are predominantly involved in our US cohort, which is similar to previous studies. The impact of damage on children’s HRQOL as perceived by parents may be different from the children’s perception and needs further examination.


Lakshmi N. Moorthy: Arthritis Foundation, 2; Maria J. Baratelli: Arthritis Foundation, 2; Margaret G.E. Peterson: Arthritis Foundation, 2; Afton L. Hassett: Arthritis Foundation, 2; Alexa B. Adams: None; Laura V. Barinstein: None; Emma J. MacDermott: None; Elizabeth C. Chalom: None; Karen Onel: None; Linda I. Ray: None; Jorge Lopez-Benitez: None; Christina Pelajo: None; Kathleen A. Haines: None; Daniel J. Kingsbury: None; Victoria W. Cartwright: None; Philip J. Hashkes: None; Nora G. Singer: None; Gina A. Montealegres: None; Ingrid Tomanova-Soltys: None; Andreas O. Reiff: None; Sandy D. Hong: None; Thomas J. A. Lehman: None.